2319. Combined Atrioventricular CMR Cine Imaging Provides High Diagnostic Accuracy to Detect Fabry’s Disease and Correlates with Disease Severity
Authors * Denotes Presenting Author
  1. Tilman Emrich *; MUSC; University Medical Center Mainz
  2. Moritz Halfmann; University Medical Center Mainz
  3. Uwe Joseph Schoepf; MUSC
  4. Basel Yacoub; MUSC
  5. Akos Varga-Szemes; MUSC
  6. Sebastian Altmann; University Medical Center Mainz
Fabry’s disease (FD) is characterized by the accumulation of sphingolipids, leading to increased myocardial mass and shortened myocardial T1. Sphingolipid accumulation results in the pathological altering of myocardial geometry and function, which correlates with adverse events (1). Data concerning the correlation and diagnostic performance comparing markers of disease severity (determined by myocardial mass and T1) and left atrial (LA) function using cardiac MRI has not been demonstrated. The aim of this study was to evaluate the changes in LA function using atrial strain imaging in correlation to disease severity and demonstrate the improvement in diagnostic performance by adding functional parameters of the LA to established markers of myocardial involvement in FD.

Materials and Methods:
Forty-nine FD patients and 50 healthy volunteers who had previously undergone cardiac MRI at 3T were included in this retrospective study. Left ventricular myocardial T1 and mass were quantified using a dedicated cardiovascular software solution. Global longitudinal strain of the LA was calculated from 2-, 3-, and 4-chamber cine acquisitions using the feature-tracking technique. Group comparisons were performed using the Mann-Whitney U test and correlation was evaluated using Pearson’s correlation coefficient. Receiver operating characteristic (ROC) curve analysis was used to calculate the area under the curve (AUC). The DeLong method was used for pairwise comparison of ROC curves to determine significant differences between AUCs. Significant difference was considered at P-values less than 0.05 on a local level.

There were significant differences between healthy volunteers and FD patients in T1 (1175 ± 50 ms vs 1124 ± 65 ms, p<0.001), myocardial mass (54.6 ± 9.8 g/m2 vs 75.2 ± 24.7 g/m2, p<0.001), and LA reservoir strain (41.6 ± 7.8 % vs 32.8 ± 10.2 %, p<0.001). Increase in left ventricular mass was inversely correlated to T1 in FD (r=-0.71, p<0.001). LA reservoir strain correlated with T1 (r=0.421, p=0.023) and inversely with left ventricular myocardial mass (r=-0.489, p<0.001). Adding parameters of left atrial function to myocardial mass improved the diagnostic performance to detect FD in every disease stage and provided an excellent diagnostic performance (AUC = 0.98, Sensitivity 97.8%, Specificity 92.0%), outperforming established parameters of myocardial involvement (e.g. Mass alone: AUC=0.80; T1: AUC= 0.60; EF: AUC=0.58; p<0.01 for all).

Atrial strain parameters have the potential to complement current multiparametric approaches in order to diagnose myocardial involvement in FD, evaluate disease severity and progression, guide initiation of medical therapy and monitor treatment success.